Background During the first year of life, and especially in the first 6 months, urinary tract infections, congenital anomalies of the kidney and urinary tract (CAKUT)—particularly obstructive types—and the coexistence of both factors may lead to transient resistance to aldosterone. This condition is characterized by fluid and salt loss despite significant activation of the renin–angiotensin–aldosterone system, and by electrolyte disturbance featuring hyponatremia, hyperkalemia, and metabolic acidosis with a normal anion gap. Transient resistance to aldosterone is not widely recognized by clinicians. Our objective was to describe the clinical presentations and management strategies in order to propose a comprehensive diagnostic approach. Methods We conducted a 13-year retrospective study involving 35 children from eight centers across France, as well as one child from Switzerland. Results Most of the children in our cohort were under 6 months of age (68.6% male). The clinical presentation of pseudohypoaldosteronism was variable and often similar to that of adrenal insufficiency. The pyelonephritis diagnosis was retained in 94.3% of the cases and uropathy was present in 85.7% of the children. Treatment was primarily symptomatic, and included intravenous hydration, salt supplementation, correction of potassium levels, and antibiotic therapy when pyelonephritis was diagnosed. Conclusion: Transient type I pseudohypoaldosteronism should be promptly considered in any infant presenting with a deteriorating general condition and fluid and electrolyte imbalances, particularly hyponatremia and hyperkalemia, in the context of acute pyelonephritis and/or uropathy.<Table Float="No" ID="Taba"> <tgroup cols="2"> <colspec align="left" colname="c1" colnum="1" /> <colspec align="left" colname="c2" colnum="2" /> <tbody> <row> <entry nameend="c2" namest="c1"> What is Known: • Transient type I pseudohypoaldosteronism affects infants under 1 year of age with congenital anomalies of the kidney and urinary tract during acute pyelonephritis. • Treatment mostly consists of antibiotics and parenteral fluids. </entry> </row> <row> <entry nameend="c2" namest="c1"> What is New: • This is the first article to report on 34 cases from a single country. • The importance of renal ultrasound in the context of urinary tract infection with salt wasting symptoms in infants without known congenital anomalies the kidney and urinary tract. • The importance of assessing hydro-electrolytic status in cases of urinary tract infection in infants under 6 months with or without known congenital anomalies of the kidney and urinary tract. • A decision tree for the diagnostic approach of transient type I pseudohypoaldosteronism. </entry> </row> </tbody> </tgroup> </Table>

Transient pseudohypoaldosteronism type I in infants with urinary tract infections and/or uropathy: insights from a French multicenter cohort

期刊论文
发表日期：

2025年11月10日

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Inès Bernhard,
Claire Dossier,
Laura Bianchi,
Cyrielle Parmentier,
Tim Ulinski,
Laurence Heidet,
Isabelle Talon,
Élise Petit,
Massimiliano Bertacchi,
Justine Perrin,
Ania Benniour,
Elise Paugam,
Sylvie Cloarec,
Violaine Lefranc,
Sylvie Rossignol,
Ariane Zaloszyc

摘要

Background

During the first year of life, and especially in the first 6 months, urinary tract infections, congenital anomalies of the kidney and urinary tract (CAKUT)—particularly obstructive types—and the coexistence of both factors may lead to transient resistance to aldosterone. This condition is characterized by fluid and salt loss despite significant activation of the renin–angiotensin–aldosterone system, and by electrolyte disturbance featuring hyponatremia, hyperkalemia, and metabolic acidosis with a normal anion gap. Transient resistance to aldosterone is not widely recognized by clinicians. Our objective was to describe the clinical presentations and management strategies in order to propose a comprehensive diagnostic approach.

Methods

We conducted a 13-year retrospective study involving 35 children from eight centers across France, as well as one child from Switzerland.

Results

Most of the children in our cohort were under 6 months of age (68.6% male). The clinical presentation of pseudohypoaldosteronism was variable and often similar to that of adrenal insufficiency. The pyelonephritis diagnosis was retained in 94.3% of the cases and uropathy was present in 85.7% of the children. Treatment was primarily symptomatic, and included intravenous hydration, salt supplementation, correction of potassium levels, and antibiotic therapy when pyelonephritis was diagnosed.

Conclusion: Transient type I pseudohypoaldosteronism should be promptly considered in any infant presenting with a deteriorating general condition and fluid and electrolyte imbalances, particularly hyponatremia and hyperkalemia, in the context of acute pyelonephritis and/or uropathy.

What is Known:

• Transient type I pseudohypoaldosteronism affects infants under 1 year of age with congenital anomalies of the kidney and urinary tract during acute pyelonephritis.

• Treatment mostly consists of antibiotics and parenteral fluids.

What is New:

• This is the first article to report on 34 cases from a single country.

• The importance of renal ultrasound in the context of urinary tract infection with salt wasting symptoms in infants without known congenital anomalies the kidney and urinary tract.

• The importance of assessing hydro-electrolytic status in cases of urinary tract infection in infants under 6 months with or without known congenital anomalies of the kidney and urinary tract.

• A decision tree for the diagnostic approach of transient type I pseudohypoaldosteronism.